CASE 6 – A CASE OF GOITER

A 52-year-old male was referred to the endocrine clinic due to fatigue, thyromegaly, and neck discomfort for 2 months. He was seen by his primary doctor for fatigue and neck discomfort and treated with antibiotics without improvement.

What are the important points in history that you would ask when dealing with a case of Goiter ?

Q What is the symptom of obstruction due to substernal or restrosternal goiter ?

Cough, stridor and dyspnea on

Lying down

Raising hand above the hand

Bending forward

Q What are complications due to obstructing goiter ?

Dyspnea – tracheal compression

Dysphagia- esophageal compression- less common

Hoarseness of voice- recurrent laryngeal nerve involvement

Orthopnea and dyspnea- phrenic nerve palsy

Horner’s syndrome

Vascular involvement

Jugular vein compression/ thrombosis

Cerebral steal syndrome

Superior vena cava obstruction

He underwent an ear, nose, and throat (ENT) evaluation and was subsequently referred to endocrinology after a diagnosis of primary hypothyroidism was made (thyroid-stimulating hormone [TSH] 65 μIU/mL, free thyroxine [T4] 0.27 ng/dL), and he was started on levothyroxine.

A diagnosis of Hashimoto thyroiditis was eventually made based on thyroid peroxidase antibodies of 498 IU/mL (0–9). He had no history of head or neck radiation. A physical exam was remarkable for thyromegaly and mild plethora of his face and upper neck, which significantly worsened upon raising his arms above his head. Additionally, he developed engorged neck veins and stridor with significant distress. (Click here)

Q What are the different methods of Palpating a Goiter ?

Pizzilo’s method- examiner stands behind the patient and uses both the hands with thumbs are the occiput

Lahey’s method- Examiner sits in front and pushes the gland towards the opposite side and palpates with the other fingers

Crile’s method- examiner uses the thumb to palpate the nodules

Q What is the maneuver described above ?

It is the Pemberton’s Maneuver

Q What is the clinical importance of the maneuver ?

It suggests a substernal goiter or a large goiter obstructing the thoracic inlet

Q How is the Pemberton’s sign carried out ?

Patient is asked to raise the hand above the head and the arms are touching the ears when raised. After about 60 seconds the patient develops facial plethora, cyanosis and discomfort. This is because of venous obstruction.

Q What the principle behind the Pemberton’s ?

Because of the substernal goiter the thorasic inlet is already narrowed. By raising the hands above the head, the inlet is narrowed further leading the patient experience symptoms of inlet obstruction .

Q What is ‘Thyroid cork’ phenomenon ?

On raising the hand and impact the thoracic inlet moves up leading to the goiter obstructing the inlet. Some say it is because of the cervical goiter moving down into the thyroid inlet. This is called ‘Thyroid cork’ phenomenon. Since there is no substernal involvement in this case, the positive Pemberton’s sign may be because of this phenomenon in this case. Flexion of the neck may also produce similar phenomenon.

Q What is the recent theory explaining the Pemberton’s sign ?

Recent theory is proposed by De Fillips et al . They propose that the acromian end of the clavicles move medially and inferiorly on movement of the hand above the head . The veins get trapped between a large relatively fixed thyroid gland and the clavicles- like a nut getting trapped between a ‘nutcracker’ .

The patient elected initial medical treatment. However, he continued to worsen clinically with progressive neck discomfort, dysphagia, a choking sensation, and engorged neck veins when combing his hair or raising his hands for other activities.

He underwent an uneventful total thyroidectomy. The specimen weighed 240 g.

Strangely no ! Pemberton’s sign tends to be more common in patients with euthyroid or hyperthyroid cases. Hypothyroidism often gets diagnosed early so the that the thyroid gland is never that large or substernal to cause the Pemberton’s sign.

Q Summarize the various theories for Pemberton’s sign ?

Narrowing of an already narrow inlet due to substernal goiter

Thyroid cork phenomenon

Ascent of the thorasic inlet

Descent of the cervical goiter

Nutcraker phenomenon – De Fillipis theory

LEARNING POINTS FROM THIS CASE

Pemberton’s sign is generally rare in Hypothyroidism due to autoimmune thyroiditis.

Pemberton’s sign can be explained by a new theory –‘Nutcracker theory’ which says that the phenomenon is because of entrapment of the external jugular vein between the large goiter and the medial end of the clavicle on movement of the hand above the head.